Diagnosis and treatment of hereditary angioedema with normal C1 inhibitor

Table 1 Treatments in hereditary angioedema with normal C1-INH (HAE type III) as reported up to now

	Treatment effective (no. of patients; [reference])	Treatment not effective (no. of patients; [reference])
Acute attacks
C1-INH concentrate	6 [14]	4 [1]
		1 [14]
Icatibant	3 [37]	0
Corticosteroids	0	23 [1]
		1 [7]
		1 [22]
Antihistamines	0	23 [1]
		1 [7]
		1 [21]
		1 [22]
Adrenalin	0	1 [21]
Prophylaxis
Androgens	2 [1]	0
	1 [3]
	1 [38]
Tranexamic acid	1 [10]	2 [1]
	1 [14]	1 [37]
Progesterone	1 [5]	0
	1 [10]
	8 [14]

ISSN: 1710-1492